Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty

Tomohiro Yamaguchi; Yasuhiro Izumiya; Hiroya Hayashi; Yoshitaka Ichikawa; Hirotoshi Ishikawa; Atsushi Shibata; Takanori Yamazaki; Minoru Yoshiyama

doi:10.1016/j.rmcr.2020.101235

Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty

Respir Med Case Rep. 2020 Sep 28:31:101235. doi: 10.1016/j.rmcr.2020.101235. eCollection 2020.

Authors

Tomohiro Yamaguchi¹, Yasuhiro Izumiya¹, Hiroya Hayashi¹, Yoshitaka Ichikawa¹, Hirotoshi Ishikawa¹, Atsushi Shibata¹, Takanori Yamazaki¹, Minoru Yoshiyama¹

Affiliation

¹ Department of Cardiovascular Medicine, Osaka City University Graduate School of Medicine, Osaka, Japan.

Abstract

A-71-year-old woman was diagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) accompanied by essential thrombocythemia (ET) with JAK2 V617F mutation. Blood test showed remarkable increase of platelet counts (132.9 × 10^4/μL) and elevated plasma BNP level (125.1pg/mL). Right heart catheterization (RHC) revealed remarkably high mean pulmonary arterial pressure (mPAP) of 43 mmHg. We gave her riociguat of 7.5mg, oral anticoagulants, oxygen inhalation for CTEPH, and anagrelide for ET. We performed 4 sessions of balloon pulmonary angioplasty (BPA) in 9 months RHC revealed successful hemodynamic improvement (mPAP = 21 mmHg) after final BPA procedure without riociguat. At six month later after final BPA procedure, RHC showed steadily improvement of mPAP (21 mmHg) without riociguat and oxygen inhalation. She lives well without oxygen inhalation and PH targeted therapy. This is the first report of successful treatment for a patient with CTEPH comorbid with ET with JAK2 V617F mutation by BPA.

Keywords: 6MWT, 6-min walking test; BNP, Brain natriuretic peptide; BPA, balloon pulmonary angioplasty; Balloon pulmonary angioplasty; CTEPH, chronic thromboembolic pulmonary hypertension; Chronic thromboembolic pulmonary hypertension; ET, essential thrombocythemia; Essential thrombocythemia; PEA, pulmonary endarterectomy; PH, pulmonary hypertension; RHC, right heart catheterization; mPAP, mean pulmonary arterial pressure.

Publication types

Case Reports